can supply home IVIg anywhere in USA & get insurance auth |
Special Google Health
Warning for children with CIDP BELOW
Progressive muscle weakness after high-dose steroids in two children
Rostasy KM, Diepold K, Buckard J, Brockmann K, Wilken B, Hanefeld F.
Department of Pediatrics and Neuropediatrics,
Georg-August-Universitat Gottingen,., Gottingen, Germany.
Corticosteroids and intravenous immunoglobulins belong to the first
line of treatment in chronic inflammatory demyelinating
polyneuropathy. In patients with a progressive course, plasma
exchange and immunomodulatory drugs are added to the regimen. To
reduce the side effects of long-term oral prednisolone, high-dose
pulsatile intravenous methylprednisolone treatment has been
advocated. We report two children with chronic inflammatory
demyelinating polyneuropathy who, after high-dose intravenous
pulsatile methylprednisolone, experienced a significant clinical
deterioration with profound loss of muscle strength. Both patients
improved after changing treatment to immunoglobulins in one and
cyclosporine combined with immunoglobulins and oral prednisolone in
Childhood chronic inflammatory demyelinating polyneuropathy:
clinical course and long-term outcome.
Ryan MM, Grattan-Smith PJ, Procopis PG, Morgan G, Ouvrier RA.
Department of Neurology, The Royal Alexandra Hospital for Children,
We reviewed the clinical history, electrophysiologic and pathologic
findings, and response to therapy of 16 children with chronic
inflammatory demyelinating polyneuropathy. The majority presented
with lower limb weakness. Sensory loss was uncommon. The illness was
monophasic in seven children, relapsing in six, and three had a
slowly progressive course. All patients were treated with
immunosuppressive agents. In 11, the initial treatment was
prednisolone. All had at least a short-term response but five went
on to develop a relapsing course. Intravenous immunoglobulin was the
initial treatment in four patients. Three responded rapidly, with
treatment being stopped after a maximum of 5 months. In resistant
chronic inflammatory demyelinating neuropathy, in addition to
prednisolone and immunoglobulin, plasma exchange, azathioprine,
cyclosporine, methotrexate, cyclophosphamide and pulse
methylprednisolone were tried at different times in different
patients. On serial neurophysiologic testing slowing of nerve
conduction persisted for long periods after clinical recovery.
Follow-up was for an average of 10 years. When last seen 14 patients
were asymptomatic, two having mild residual deficits. Childhood
chronic inflammatory demyelinating neuropathy responds to
conventional treatment and generally has a favourable long-term
Childhood chronic inflammatory demyelinating polyneuropathy.
The Institute for Child Development, Division of Pediatrics, Dana
Children's Hospital, Sackler School of Medicine, Tel Aviv
Chronic inflammatory demyelinating polyneuropathy (CIDP) is a
chronic disorder of the peripheral nervous system with sensory and
motor involvement, and insidious onset over a period of months. In
children and adults, both proximal and distal muscles are affected.
Muscle stretch reflexes are absent or depressed. Laboratory findings
include elevated cerebrospinal fluid protein with no increase of
mononuclear cells. Electrophysiological and pathological studies
show evidence of demyelination. No control studies of the efficacy
of immunomodulating therapy in childhood CIDP are available.
However, several studies have indicated clinical improvement after
treatment with prednisolone, plasmapheresis and intravenous
immunoglobulin, but disappointing results with other
immunosuppressive agents. While some children have a monophasic
course, with complete recovery, others have a protracted course,
with either a slowly progressive or a relapsing-remitting course,
resulting in prolonged morbidity and disability.
Chronic inflammatory demyelinating polyneuropathy in childhood.
Department of Neurology, St. Louis Children's Hospital, Washington
University of Medicine, St. Louis, Missouri 63110, USA.
Chronic inflammatory demyelinating polyneuropathy (CIDP) in children
is relatively rare. However, it has been recognized for many years.
In patients presenting with this disease, subacute onset of weakness
usually develops over at least 2 months and often progresses to a
loss of ambulation. Some children's initial presentations may mimic
Guillain-Barre syndrome. Dysasthesias are common. Males are affected
more than females, and antecedent illnesses or vaccinations occur in
approximately half of patients.
Physical examination reveals
diffuse, proximal greater than distal weakness, with an absence or
depression of muscle stretch reflexes. Electrophysiology confirms demyelination, and spinal fluid examination demonstrates
albuminocytologic dissociation. The clinical presentation,
diagnosis, and prognosis of childhood CIDP are reviewed. Treatment
and immunologic features are also discussed in this article.
Please continue to information on
EMG/NCV next page